Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P04637 (p53)
 77,613 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hibernomas are rare neoplasms composed of brown adipose tissue. The behavior of these neoplasms has been described as uniformly benign in humans. The only recurrence cited in the English literature involved a sarcoma with hibernoma-like features, which was reported in abstract form. We present 2 cases of hibernoma, one that continued to grow following partial excision and another at an unusual site (anterior abdominal wall). Both of these tumors overexpressed p53 protein by immunohistochemistry, which was a novel finding. A review of the literature highlights recent advances that may help confirm the diagnosis and explain the biology of these rare tumors.
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PMID:Hibernoma: a report of 2 unusual cases with a review of the literature. 1217

Hibernoma is a rare benign adipocytic tumor composed of a proliferation of brown and white fat cells varying in their proportions. The tumor may also contain fat cells resembling lipoblasts, which makes it difficult to distinguish it from atypical lipomatous tumor/well differentiated liposarcoma (ALT/WDLS). Although nuclear expressions of murine double minute 2 (MDM2) and cyclin-dependent kinase 4 (CDK4) are widely used as immunohistochemical surrogate markers for ALT/WDLS, the utility of these proteins in distinguishing between hibernoma and ALT/WDLS still remains to be elucidated. We evaluated immunohistochemical expressions of MDM2 and CDK4 in 10 hibernomas expressing uncoupling protein-1 (UCP-1), a mitochondrial protein transporter consistently expressed in brown fat cells, and lacking MDM2 gene amplification, which was analyzed by fluorescence in situ hybridization (FISH). In contrast to the data previously obtained, nuclear expression of MDM2 was observed in 100% (10/10 cases) of the hibernomas irrespective of the proportion of brown fat cells, whereas no cases were positive for CDK4. The tumors also showed almost concurrent expression of p53 (in 9/10 cases) and ubiquitin-specific-processing protease 7 (USP7) (in 10/10 cases), which deubiquitinates and stabilizes MDM2, potentially resulting in its nuclear expression without MDM2 gene amplification. MDM2 expression may thus be a diagnostic pitfall for hibernoma particularly in differentiating it from ALT/WDLS.
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PMID:Nuclear expression of MDM2 in hibernoma: a potential diagnostic pitfall. 3281 35